糖尿病性浮腫性硬化症(<特集>皮膚疾患とアフェレシス)

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Diabetic scleredema (DS) has been reported to occur in 2.5-14% of diabetic patients. A few patients are severe cases with considerable restriction in the activities of daily living (ADL) Moreover, there is no effective method established for the treatment of DS. We introduced low density lipoprotein (LDL) apheresis combined with an anti-diabetic drug and a cholesterol-lowering drug to the treatment of severe DS in diabetic patients complicated with familial hypercholesterolemia (FH). DS symptoms were improved gradually as a result of the LDL apheresis performed once weekly. As the symptoms mostly disappeared and the conditions were stabilized, the frequency of LDL apheresis was reduced to once biweekly with course monitoring. However, similar DS symptoms recurred 6 years later. Thus, the LDL apheresis was increased to once weekly like the time of initial onset without changing the oral drugs or insulin dosage, which led to prompt improvement of DS symptoms. The skin biopsy findings included thickening of the dermis and collagen bundles, and deposition of strong acid mucopolysaccharides and accumulation of LDL between collagen bundles. These findings decreased after the treatment. These results suggest effectiveness of the LDL apheresis combined with a drug therapy for skin lesions in severe DS patients. The LDL apheresis should be performed in severe DS patients from now on in order to examine its effects on the disease.
日本アフェレシス学会の論文
2008-05-31

糖尿病性浮腫性硬化症(<特集>皮膚疾患とアフェレシス)

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